Unilateral vasectomy for intractable epididiymo-orchitis in patients with anorectal malformation.

BACKGROUND: Male patients treated for anorectal malformations (ARM) and recto-urethral fistula (RUF) tend to develop recurrent epididymo-orchitis (EO) which occurs approximately in 20% of all them. The optimal management of this condition is unclear because of the extreme its rarity and the unavailability of detailed analysis in literature. To date the majority of this patients benefits from medical treatment and symptoms reduce over time but few data have been published in literature about management of patients with intractable EO. OBJECTIVE: To describe the efficacy of unilateral vasectomy in patients operated on for anorectal malformations with RUF and affected by intractable EO. STUDY DESIGN: We present five patients who met the criteria for intractable EO, and followed at our centre four of whom have undergone unilateral vasectomy. RESULTS: The first episode of EO presented at 42,00 mos ±29.39. Initially, patients were all managed with analgesics and antibiotics. For the failure of therapy, five patients were all offered unilateral vasectomy but only four families accepted procedure. Surgical treatment was performed as a day case without complications. Postoperative follow up was 88,50 mos ±68.36. Prompt and durable resolution of symptoms was observed. DISCUSSION: The long-term effects of recurrent EO in ARM are often underestimated. Prompt and appropriate intervention should prevent this undesirable sequela. Unfortunately, the optimal management of this complication is unclear, partly because of its extreme rarity. The established management needs to follow the route of correcting underlying anomalies and providing long-term analgesic and antibiotics but this may have undesired side effects. We therefore offered families vasectomy for complete symptom resolution and/or drug withdrawal. Vasectomy, as a form of treatment for, can be justified if it can prevent pain, infection and destruction of the testes. Early vasectomy may save enough functional testis tissue. CONCLUSION: To date, the only available treatment to achieve definitive resolution of symptoms in intractable unilateral EO is vasectomy. Long-term effects of such procedure on fertility are unknown. The treatment of recurrent EO in cases without site predilection remains a matter of contention.

Branchial cleft and pouch anomalies in childhood: a report of 50 surgical cases.

BACKGROUND AND AIMS: Branchial abnormalities occur when there is disturbance in the maturation of the branchial apparatus during fetal development. Branchial anomalies are congenital lesions usually present in childhood, even if they can be diagnosed later for enlargement or infection. A correct diagnosis will lead to proper management: complete surgical excision is the treatment of choice. The purpose of this article is to present clinical features, diagnostic methods and surgical treatment of branchial anomalies in childhood, based on a series of 50 patients. METHODS: We conducted a retrospective analysis of a total of 50 pediatric patients operated from June 2005 to June 2014 for the presence of branchial cleft anomalies. RESULTS: 27 cases (54 %) presented a second branchial cleft fistula and 11 cases (22 %) a second branchial cleft cyst and one case (2 %) presented both cyst and sinus of the second branchial cleft; four cases (8 %) presented first branchial cleft cyst whereas four cases (8 %) a first branchial cleft sinus and two cases (4 %) a first branchial cleft fistula; one case (2 %) presented a piriform sinus fistula (third branchial cleft). None of our patients presented anomalies of the fourth branchial cleft. All patients underwent surgical treatment and lesions have been removed by excision or fistulectomy. No post-surgical complication occurred. The rate of recurrence was 4 %. CONCLUSIONS: Pre-operative diagnosis supplies important information to the surgeon for a proper therapy: a complete excision of the lesion without inflammatory signs is essential to avoid re-intervention and to achieve a good outcome.

Muscle thickness in infants hypertrophic pyloric stenosis.

Hypertrophic pyloric stenosis (IHPS) is the most common abdominal abnormality requiring surgery in infants. It occurs due to the hypertrophic and hyperplasia of the muscular layers of the pyloric. The usual age of clinical presentation is about three weeks of life. The most important symptom is non bilious emesis, intermittent or after each feeding. From march 1996 to June 2001, 21 infants, 20 males and 1 female, were subjected to ultrasonographic, radiographic exams and after diagnosis to the pyloromyotomy extramucosa. Ultrasonography was the study of choice used to identify hypertrophic pyloric stenosis; the markers to analyse were the length and the overall diameter of the pyloric canal and the muscle thickness of the wall. The results showed that a length of the pyloric canal 20 +/- 6 mm, a diameter 13.6 +/- 2.5 mm and a muscle thickness 4.1 +/- 1 mm are diagnostics for hypertrophic pyloric stenosis.

Graves-Basedow's disease in children and adolescents: total vs subtotal thyroidectomy.

"Graves' disease" is an autoimmune pathology and is the most important cause of hyperthyroidism in children and adolescents. The treatment for this disease is controversial; there are three different options: synthetic antithyroid drugs, ablation of the thyroid gland with I 131 or surgery. Our study reports the clinical and therapeutic aspects and long-term results of 27 patients (< = 18 years old) treated with total thyroidectomy or "near-total" thyroidectomy from 1975 to August 2001. Antithyroid drugs represent the preliminary treatment of choice. For a long time in the past therapy with radioactive iodine was not recommended; now it represents an efficiently therapy with low morbility. Surgical treatment is suitable when a young patient presents a considerable enlargement of the thyroid gland or when antithyroid medical treatment does not achieve a good control of the disease, or when rapid control of hyperthyroidism is necessary. The surgical treatment can be a total thyroidectomy or a "near-total" thyroidectomy. The first is the best choice for us, because it has a low risk of relapse and a major efficacy in the ophthalmopathy.

[Prophylactic total thyroidectomy in children and adolescents with genetic mutations in the RET-protooncogene]. / La tiroidectomia totale profilattica nei bambini e negli adolescenti portatori di mutazioni genetiche nel proto-oncogene RET.

Medullary thyroid cancer (C.M.T.) can be a sporadic form generally in adults or a heredofamilial form where the first symptom appears in pediatric and adolescent age. The hereditary form can be isolated or associated with others endocrine neoplasias of type 2: MEN2a (with or without cutaneous lichen amyloidosis) and MEN2b. The responsible gene of the transmission has been identified in proto-oncogene RET localized on chromosome 10. Point form mutations of this proto-oncogene have been found on exons 10 and 11 in MEN2a and on 16 in MEN2b. In our study on 64 subjects, who belong 11 familiar groups, affected by MEN2a, MEN2b and familiar C.M.T., underwent a genetic research to look for point form mutations of proto-oncogene RET with PCR followed by the analysis of restriction. A genetic mutation has been revealed in 25 subjects: 18 were already known affected by MEN2 and so surgical treated and 7 seemed healthy (mean age 17.4 years, range 10-25). These 7 patients has been undergone clinical research and surgical treatment: a total thyroidectomy associated a lymphectomy of the central compartment. In all cases the histological exam showed C.M.T. moreover a patient had metastasis in lymph nodes of the central compartment. Another had hyperparathyroidism and pheochromocytoma treated with total thyroidectomy, parathyroidectomy and bilateral laparoscopic adrenalectomy. The identification in a very early age of carrier subjects of hill's gene inside an affected family, permits the execution of a prophylactic total thyroidectomy to prevent the C.M.T.. The penetrance of this neoplasia in hereditary form is 100%.